하멜 漂流記에 나타난 李朝中葉의 佛敎像

洪知吾(홍지오) 동국대학교 석림회 1973 석림 Vol.6ㆍ7 No.-

Complete Recovery of Oxysterol 7α-Hydroxylase Deficiency by Living Donor Transplantation in a 4-Month-Old Infant: the First Korean Case Report and Literature Review

홍지나,오석희,유한욱,Hiroshi Nittono,Akihiko Kimura,김경모 대한의학회 2018 Journal of Korean medical science Vol.33 No.51

Oxysterol 7α-hydroxylase deficiency is a very rare liver disease categorized as inborn errors of bile acid synthesis, caused by CYP7B1 mutations. As it may cause rapid progression to end-stage liver disease even in early infancy, a high index of suspicion is required to prevent fatal outcomes. We describe the case of a 3-month-old boy with progressive cholestatic hepatitis and severe hepatic fibrosis. After excluding other etiologies for his early liver failure, we found that he had profuse urinary excretion of 3β-monohydroxy-Δ5 -bile acid derivatives by gas chromatography/mass spectrometry analysis with dried urine spots on filter paper. He was confirmed to have a compound heterozygous mutation (p.Arg388Ter and p.Tyr469IlefsX5) of the CYP7B1 gene. After undergoing liver transplantation (LT) from his mother at 4 months of age, his deteriorated liver function completely normalized, and he had normal growth and development until the current follow-up at 33 months of age. We report the first Korean case of oxysterol 7α-hydroxylase deficiency in the youngest infant reported to undergo successful living donor LT to date.

만성 소화기 질환 환아에서 미량원소 결핍과 모발 검사의 유용성

홍지나,이정화,이란,신지연,고재성,서정기,Hong, Jea-Na,Lee, Jung-Hwa,Lee, Ran,Shin, Jee-Youn,Ko, Jae-Sung,Seo, Jeong-Kee 대한소아소화기영양학회 2008 Pediatric gastroenterology, hepatology & nutrition Vol.11 No.2

목 적: 만성 설사, 흡수 장애 등의 소화기 질환에서 경구 영양 공급이 불충분할 경우 미량원소 결핍 발생의 빈도가 높으나, 이러한 환아에서 미량원소 결핍 증상에도 불구하고 혈액 검사 결과 정상으로 나타나기도 한다. 따라서 미량원소 결핍 고위험군 환아에서 결핍된 미량원소를 확인하고, 적절한 공급 및 추적 관찰 지표로서 모발 검사의 유용성을 알아보고자 하였다. 방 법: 서울대학교 어린이병원에 내원하여 만성 소화기 질환으로 경구 영양 장애 또는 성장 발육 부전을 보였던 13명을 대상으로 모발과 혈액의 미량원소 검사를 시행하여, 결핍 소견을 보인 모발과 혈액의 미량원소에 대해 장기 정맥영양군과 경구영양군, 그리고 증상군과 무증상군으로 나누어 비교, 분석하였다. 결 과: 전체 13명 중 11명에서 모발 또는 혈액 검사에서 미량원소 결핍이 있었으며, 결핍된 미량원소는 아연, 셀레늄, 구리였다. 모발 내 아연 결핍은 8명(62%), 모발 내 셀레늄 결핍은 6명(46%)이었고, 혈액 내 아연농도가 정상인 환자의 67% (8/12명), 혈액 내 셀레늄 농도가 정상 환자의 57% (4/7명)에서 각각 모발 내 아연, 모발 내 셀레늄 결핍을 보였다. 모발 내 아연 농도는 장기 정맥영양군에서 경구영양군에 비해 유의하게 낮았으며(p=0.015) 결핍 발생 빈도 또한 유의하게 높았다(Fisher's exact test, p=0.032). 모발 내 셀레늄 농도는 증상군에서 무증상군에 비해 유의하게 낮았다(p=0.034). 결 론: 만성 소화기 질환으로 경구 영양 장애 또는 성장 발육 부전이 있을 경우 증상 여부에 상관없이 혈액검사와 함께 모발 미네랄 검사로 결핍 여부를 평가하는 것이 필요할 것으로 보인다. Purpose: Patients with chronic gastrointestinal disease are at risk for trace element deficiency due to impaired absorption and gastrointestinal loss. The aim of this study was to evaluate the trace element status of patients with gastrointestinal disease by blood and hair analysis, and to determine the usefulness of hair mineral analysis for diagnosing trace element deficiency not detected by a blood test. Methods: An analysis of hair minerals was performed and compared with blood mineral analysis in 13 patients with chronic gastrointestinal disease. The concentration of each element in the hair and blood was compared in the subgroups based on parenteral nutritional support or clinical symptoms. Results: Almost all patients had trace element deficiency. The trace elements deficient in the blood or hair analysis included zinc, selenium and copper. The hair zinc concentration was significantly lower in the group receiving parenteral nutritional support. The hair selenium concentration was statistically associated with the clinical symptoms of hair loss, brittle hair and loss of hair pigmentation. Conclusion: The results of this study suggest that patients with chronic gastrointestinal disease should receive adequate zinc and selenium replacement to avoid trace element deficiency especially when treated with long-term parenteral nutrition. Hair mineral analysis is useful as a complementary tool for the detection of a trace element deficiency.

Laboratory Markers Indicating Gastrointestinal Involvement of Henoch-Schönlein Purpura in Children

홍지나,양혜란 대한소아소화기영양학회 2015 Pediatric gastroenterology, hepatology & nutrition Vol.18 No.1

Purpose: To determine clinically useful biochemical markers reflecting disease activity and/or gastrointestinal (GI) tract involvement in Henoch-Schönlein purpura (HSP). Methods: A total of 185 children with HSP and 130 controls were included. Laboratory data indicating inflammation, standard coagulation, and activated coagulation were analyzed for the HSP patients, including measurements of the hemoglobin level, white blood cell (WBC) count, absolute neutrophil count (ANC), platelet count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, prothrombin time, activated partial thromboplastin time, and fibrinogen, D-dimer, and fibrin degradation product (FDP) levels. The clinical scores of the skin, joints, abdomen, and kidneys were assessed during the acute and convalescence phases of HSP.Results: The WBC count, ANC, ESR, and CRP, fibrinogen, D-dimer, and FDP levels were significantly higher in the acute phase compared with the convalescent phase of HSP (p<0.05). The total clinical scores were more strongly correlated with the D-dimer (r=0.371, p<0.001) and FDP (r=0.369, p<0.001) levels than with inflammatory markers, such as the WBC count (r=0.241, p=0.001), ANC (r=0.261, p<0.001), and CRP (r=0.260, p<0.001) levels. The pa-tients with GI symptoms had significantly higher ANC (median [interquartile range], 7,138.0 [4,446.4-9,470.0] vs. 5,534.1 [3,263.0-8,153.5], p<0.05) and CRP (0.49 [0.15-1.38] vs. 0.23 [0.01-0.67], p<0.05), D-dimer (2.63 [1.20-4.09] vs. 1.75 [0.62-3.39]), and FDP (7.10 [0.01-13.65] vs. 0.10 [0.01-7.90], p<0.05) levels than those without GI symptoms.Conclusion: D-dimer and FDPs are more strongly associated with disease activity and more consistently reflect GI involvement than inflammatory markers during the acute phase of HSP.

Clinical Applications of Gastrointestinal Manometry in Children

홍지나 대한소아소화기영양학회 2014 Pediatric gastroenterology, hepatology & nutrition Vol.17 No.1

Manometry is a noninvasive diagnostic tool for identifying motility dysfunction of the gastrointestinal tract. Despite the great technical advances in monitoring motility, performance of the study in pediatric patients has several limi-tations that should be considered during the procedure and interpretation of the test results. This article reviews the clinical applications of conventional esophageal and anorectal manometries in children by describing a technique for performing the test. This review will develop the uniformity required for the methods of performance, the parame-ters for measurement, and interpretation of test results that could be applied in pediatric clinical practice.

하멜 漂流記에 나타난 李朝中葉의 佛敎像

洪知吾(홍지오) 동국대학교 석림회 1971 석림 Vol.5 No.-

Autoimmune hepatitis-primary sclerosing cholangitis overlap syndrome in a 10-year-old girl with ulcerative colitis

홍지나,송미경,고재성,강경훈,김우선,서정기 대한소아청소년과학회 2009 Clinical and Experimental Pediatrics (CEP) Vol.52 No.4

Autoimmune hepatitis (AIH), primary sclerosing cholangitis (PSC), and primary biliary cirrhosis (PBC) are chronic autoimmune liver diseases. Overlap syndrome is defined as a condition in which the clinical, biochemical, and histological features of these autoimmune diseases are overlapped. Thus, it is difficult to appreciate overlap syndrome as an actual diagnostic entity. Only a few cases of the overlap syndrome of AIH and PSC have been reported, especially in children. Moreover, PSC is known to be the most frequent liver disorder associated with inflammatory bowel diseases such as ulcerative colitis. We report one case of AIH-PSC overlap syndrome in a child who was diagnosed as having ulcerative colitis.

讀書論

洪知吾(홍지오) 동국대학교 석림회 1974 석림 Vol.8 No.-

Breastfeeding and Red Meat Intake Are Associated with Iron Status in Healthy Korean Weaning-age Infants

홍지나,장주영,신수,오소희 대한의학회 2017 Journal of Korean medical science Vol.32 No.6

The present study investigated risk factors for iron deficiency (ID) and iron deficiency anemia (IDA) during late infancy, including feeding type and complementary feeding (CF) practice. Healthy term Korean infants (8–15 months) were weighed, and questionnaires regarding delivery, feeding, and weaning were completed by their caregivers. We also examined levels of hemoglobin, serum iron/total iron-binding capacity, serum ferritin, and mean corpuscular volume (MCV). Among 619 infants, ID and IDA were present in 174 infants (28.1%) and 87 infants (14.0%), respectively. The 288 infants with exclusively/mostly breastfeeding until late infancy (BFL) were most likely to exhibit ID (53.1%) and IDA (28.1%). The risk of ID was independently associated with BFL (adjusted odds ratio [aOR], 47.5; 95% confidence interval [CI], 18.3–122.9), male sex (aOR, 1.9; 95% CI, 1.2–2.9), fold weight gain (aOR, 2.6; 95% CI, 1.5–4.6), and perceived inadequacy of red meat intake (aOR, 1.7; 95% CI, 1.0–2.7). In addition to the risk factors for ID, Cesarean section delivery (aOR, 1.9; 95% CI, 1.1–3.2) and low parental CF-related knowledge (aOR, 2.8; 95% CI, 1.5–5.2) were risk factors for IDA. In conclusion, prolonged breastfeeding and perceived inadequacy of red meat intake may be among the important feeding-related risk factors of ID and IDA. Therefore, more meticulous education and monitoring of iron-rich food intake, such as red meat, with iron supplementation or iron status testing during late infancy if necessary, should be considered for breastfed Korean infants, especially for those with additional risk factors for ID or IDA.

한-미 FTA 추진 현황과 기대효과

홍지인,Hong, Ji-In 전국농업기술자협회 2006 농업기술회보 Vol.43 No.2